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Rabbit Anti-Tyrosine Hydroxylase/APC Conjugated antibody (bs-20080R-APC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-20080R-APC
英文名称1 Rabbit Anti-Tyrosine Hydroxylase/APC Conjugated antibody
中文名称 APC标记的酪氨酸羟化酶抗体
别    名 Tyrosine Hydroxylase; DYT14; DYT5b; ple; Protein Pale; c; The; TYH; Tyrosine 3 hydroxylase; Tyrosine 3 monooxygenase; Tyk2; TY3H_HUMAN; Tyrosine 3-monooxygenase; Tyrosine 3-hydroxylase; TH.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  免疫学  神经生物学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse, Rat, 
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 60kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Tyrosine Hydroxylase
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
The protein encoded by this gene is involved in the conversion of tyrosine to dopamine. It is the rate-limiting enzyme in the synthesis of catecholamines, hence plays a key role in the physiology of adrenergic neurons. Mutations in this gene have been associated with autosomal recessive Segawa syndrome. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]

Function:
Plays an important role in the physiology of adrenergic neurons.

Tissue Specificity:
Mainly expressed in the brain and adrenal glands.

DISEASE:
Defects in TH are the cause of Segawa syndrome autosomal recessive (ARSEGS) [MIM:605407]. A form of DOPA-responsive dystonia presenting in infancy or early childhood. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Some cases present with parkinsonian symptoms in infancy. Unlike all other forms of dystonia, it is an eminently treatable condition, due to a favorable response to L-DOPA.
Note=May play a role in the pathogenesis of Parkinson disease (PD). A genome-wide copy number variation analysis has identified a 34 kilobase deletion over the TH gene in a PD patient but not in any controls.

Similarity:
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.

Database links:

Entrez Gene: 7054 Human

Entrez Gene: 21823 Mouse

Entrez Gene: 25085 Rat

Omim: 191290 Human

SwissProt: P07101 Human

SwissProt: P24529 Mouse

SwissProt: P04177 Rat

Unigene: 435609 Human

Unigene: 1292 Mouse

Unigene: 11082 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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